Neuroendocrine Neoplasm -- Tumors whose cells possess secretory granules and originate from the neuroectoderm, i.e., the cells of the ectoblast or epiblast that program the neuroendocrine system. Common properties across most neuroendocrine tumors include ectopic hormone production (often via APUD CELLS), the presence of tumor-associated antigens, and isozyme composition.
Neuroendocrine Carcinoma -- A group of carcinomas which share a characteristic morphology, often being composed of clusters and trabecular sheets of round "blue cells", granular chromatin, and an attenuated rim of poorly demarcated cytoplasm. Neuroendocrine tumors include carcinoids, small ("oat") cell carcinomas, medullary carcinoma of the thyroid, Merkel cell tumor, cutaneous neuroendocrine carcinoma, pancreatic islet cell tumors, and pheochromocytoma. Neurosecretory granules are found within the tumor cells. (Segen, Dictionary of Modern Medicine, 1992)
Stanford University School of Medicine, 300 Pasteur Drive, Stanford, California, USA. [email protected]
Section of Gastrointestinal Surgery and the University of California, Los Angeles, USA.
Neuroendocrine tumors of the pancreas can be managed surgically with excellent outcomes.
Retrospective case series.
Academic medical center.
Seventy consecutive patients who underwent resection for pancreatic neuroendocrine tumors between January 1, 1990, and December 31, 2005.
Pancreaticoduodenectomy, distal pancreatectomy, or enucleation.
Postoperative morbidity, mortality, and long-term survival.
Of the 70 patients, 50 (71.4%) had nonfunctional tumors. Thirty-seven patients (52.9%) had neuroendocrine carcinomas and 13 (18.6%) had benign islet cell neoplasms. Twenty patients had functional tumors. Of these 20 patients, 16 had insulinomas, 2 had glucagonomas, and 2 had gastrinomas. Twenty-seven patients underwent pancreaticoduodenectomy, 32 had distal pancreatectomy, and 11 underwent enucleation. Patients undergoing enucleation as compared with those not undergoing enucleation were younger (mean age, 39 vs 51 years, respectively; P = .009) and had smaller tumors (mean tumor size, 2 vs 5 cm, respectively; P<.001). Postoperative complications occurred in 13 patients (48.1%) after pancreaticoduodenectomy, in 4 patients (12.5%) after distal pancreatectomy, and in 0 patients after enucleation. There were no perioperative mortalities. With a median follow-up of 50 months, the 5-year actuarial survival for the patients with malignant neuroendocrine carcinomas (n = 37) was 77%, and all of the patients with functional tumors are alive. The presence of lymphovascular invasion closely approached significance when survival was evaluated (P = .06). Lymph node status, perineural invasion, and liver metastasis did not impact survival.
This single-institutional case series demonstrates that pancreatic neuroendocrine tumors can be safely resected without mortality and with minimal morbidity. The presence of lymphovascular invasion can be used to classify neuroendocrine tumors as malignant, and this appears to predict survival. Patients with malignant tumors can expect long-term survival even in the setting of metastatic disease.
Department of Pancreatic Surgery, Cancer Hospital of Tianjin Medical University Key Laboratory of Cancer Prevention and Therapy, Tianjin, China.
To review the clinical data of a group of patients with pancreatic neuroendocrine tumors (pNETs) and to investigate the role of surgery in the treatment for pNETs by analyzing clinical manifestations and postoperative course of this rare disease.
A total of 112 patients (aged 21-76 years; 45 males) who underwent treatment between 1980 and 2003 were recruited in this study. Patients' data related to demographics and characteristics, diagnostic studies, surgical and tumor characteristics and survival were retrospectively reviewed.
Forty-six patients (41.1%) had a well-differentiated neuroendocrine tumor (WDT), 44 (48.2%) a well-differentiated neuroendocrine carcinoma (WD-Ca) and 12 (10.7%) a poorly differentiated neuroendocrine carcinoma (PD-Ca). Nonfunctional tumors were seen in 65 (58.0%) patients, whereas functional tumors were found in 47 (42.0%) patients, including 26 insulinomas, 17 gastrinomas, 2 VIPomas, 1 glucagonoma, and 1 ACTHoma. The sensitivity of computed tomography was 87.1%. Surgical resection was performed in 99 (88.4%) patients. Thirty-eight (33.9%) patients underwent partial pancreaticoduodenectomy, 32 (28.6%) had distal pancreatectomy and 29 (25.9%) underwent enucleation. No surgery-related death occurred. The common postoperative complications were pancreatic fistula (15.2%), wound infection (13.4%) and delayed gastric emptying (6.3%). Three (5%) patients had reoperation due to intra-abdominal abscess and postoperative hemorrhage. Twenty-six (55.3%) of the 47 functional tumors were malignant, whereas 40 (61.5%) of the 65 nonfunctional tumors were malignant. Survival was significantly related to the type of neuroendocrine tumor (p = 0.001). The overall 5-year actual survival rate of patients with WD-Ca (n = 54) was 56%, significantly less than that of patients with WDT (n = 46, 91%, p = 0.001). All the patients of PD-Ca (n = 12) group died in 5 years. The 5-year survival rate differed significantly between patients with tumor node metastasis (TNM) stage I and II disease and those with stage III and IV tumors (p = 0.011). Patients with stage III had better prognosis than those with stage IV tumors (p = 0.007). Patients' long-term survival was closely correlated with vascular invasion (p = 0.008) and resection margin (p = 0.004).
PNETs can be safely resected. Microscopic vascular invasion and positive resection margin are helpful for predicting patient survival. Malignant cases should be treated with aggressive radical surgery to achieve complete tumor resection and potential for long-term survival.
Ito H, Abramson M, Ito K, Swanson E, Cho N, Ruan DT, Swanson RS, Whang EE.
Department of Surgery, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA, USA.
The aims of this study were to evaluate contemporary outcomes associated with the surgical management of pancreatic neuroendocrine tumors (PNETs) and to assess the prognostic value of the World Health Organization (WHO) classification and TNM staging for PNETs.
The medical records of 73 consecutive patients with PNETs treated at a single institution from January 1992 through September 2006 were reviewed. Survival was analyzed with the Kaplan-Meier method (median follow-up: 43 months).
Median patient age was 52 years (range, 19-83 years), and 36 (49%) patients were male. Thirty-three patients had a well-differentiated neuroendocrine tumor (WDT), 26 had a well-differentiated neuroendocrine carcinoma (WDCa), and 14 had a poorly differentiated neuroendocrine carcinoma (PDCa). Fifty (68%) patients underwent potentially curative resection, and the 5-year disease-specific survival (DSS) rate for the entire cohort was 62%. WHO classification and TNM staging system provided good prognostic stratification of patients; 5-year DSS rates were 100% for WDT, 57% for WDCa, 8% for PDCa, respectively, by WHO classification (p < 0.001), and 100% for stage 1, 90% for stage 2, 57% for stage 3, and 8% for stage 4, respectively, by TNM stage (p < 0.001). Among the patients who underwent potentially curative resection, nodal status, distant metastasis, and tumor grade were significant prognostic factors.
WHO classification and TNM staging are useful for prognostic stratification among patients with PNETs.
Yao JC, Eisner MP, Leary C, Dagohoy C, Phan A, Rashid A, Hassan M, Evans DB.
Department of Gastrointestinal Medical Oncology, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030, USA. [email protected]
We examine the epidemiology, natural history, and prognostic factors that affect the duration of survival for islet cell carcinoma by using population-based registries.
The Surveillance, Epidemiology, and End Results (SEER) Program database (1973-2003 release, April 2006) was used to identify cases of islet cell carcinoma by histology codes and tumor site.
A total of 1310 (619 women and 691 men) cases with a median age of 59 years were identified. The annual age-adjusted incidence in the periods covered by SEER 9 (1973-1991), SEER 13 (1992-1999), and SEER 17 (2000-2003) were .16, .14, and .12 per 100,000, respectively. The estimated 28-year limited-duration prevalence on January 1, 2003, in the United States was 2705 cases. Classified by SEER stage, localized, regional, and distant stages corresponded to 14%, 23%, and 54% of cases. The median survival was 38 months. By stage, median survival for patients with localized, regional, and distant disease were 124 (95% CI, 80-168) months, 70 (95% CI, 54-86) months, and 23 (95% CI, 20-26) months, respectively. By multivariate Cox proportional modeling, stage (P < .001), primary tumor location (P = .04), and age at diagnosis (P < .001) were found to be significant predictors of survival.
Islet cell carcinomas account for approximately 1.3% of cancers arising in the pancreas. Most patients have advanced disease at the time of diagnosis. Despite the disease's reputation of being indolent, survival of patients with advanced disease remains only 2 years. The development of novel therapeutic approaches is needed.
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